About The Medical Condition Debrancher Deficiency
Find the definition of the medical term Debrancher Deficiency. Debrancher Deficiency defined and explained for easy understanding at the Medical Conditions from ClusterMed.info.
Debrancher Deficiency
| Debrancher Deficiency Medical Condition |
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What's The Definition Of The Medical Condition Debrancher Deficiency?
An autosomal recessive metabolic disorder due to deficient expression of amylo-1,6-glucosidase (one part of the glycogen debranching enzyme system). The clinical course of the disease is similar to that of glycogen storage disease type I, but milder. Massive hepatomegaly, which is present in young children, diminishes and occasionally disappears with age. Levels of glycogen with short outer branches are elevated in muscle, liver, and erythrocytes. Six subgroups have been identified, with subgroups Type IIIa and Type IIIb being the most prevalent.
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